在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

Gba Antibody, HRP conjugated

  • 中文名稱:
    Gba兔多克隆抗體, HRP偶聯
  • 貨號:
    CSB-PA009289HB01MO
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Mus musculus (Mouse) Gba Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    Gba
  • 別名:
    GbaLysosomal acid glucosylceramidase antibody; Lysosomal acid GCase antibody; EC 3.2.1.45 antibody; Acid beta-glucosidase antibody; Beta-glucocerebrosidase antibody; Cholesterol glucosyltransferase antibody; SGTase antibody; EC 2.4.1.- antibody; Cholesteryl-beta-glucosidase antibody; EC 3.2.1.104 antibody; D-glucosyl-N-acylsphingosine glucohydrolase antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Mouse
  • 免疫原:
    Recombinant Mouse Glucosylceramidase protein (20-515AA)
  • 免疫原種屬:
    Mus musculus (Mouse)
  • 標記方式:
    HRP
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    >95%, Protein G purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA
  • Protocols:
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能:
    Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramide/GlcCer into free ceramide and glucose. Thereby, plays a central role in the degradation of complex lipids and the turnover of cellular membranes. Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation. Also plays a role in cholesterol metabolism. May either catalyze the glucosylation of cholesterol, through a transglucosylation reaction that transfers glucose from glucosylceramide to cholesterol. The short chain saturated C8:0-GlcCer and the mono-unsaturated C18:0-GlcCer being the most effective glucose donors for that transglucosylation reaction. Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl-beta-D-glucoside to ceramide. Finally, may also hydrolyze cholesteryl-beta-D-glucoside to produce D-glucose and cholesterol.
  • 基因功能參考文獻:
    1. Thus, while the underlying mechanism is not clear, this model shows that gba deficiency impacts the age of onset and disease duration in aged SNCA(A53T) mice, providing a valuable resource to identify modifiers, pathways and possible moonlighting roles of glucocerebrosidase in Parkinson pathogenesis. PMID: 29173981
    2. The data support the contention that prolonged antagonism of glucosylceramide synthase (GCS)in the central nervous system (CNS)can affect alpha-synuclein processing and improve behavioral outcomes. Hence, inhibition of GCS represents a disease-modifying therapeutic strategy for GBA-related synucleinopathies and conceivably for certain forms of sporadic disease PMID: 28223512
    3. These results indicate that Gba1 deficiency enhances neuronal vulnerability to neurodegenerative processes triggered by increased alpha-synuclein expression. PMID: 28969384
    4. This study demonstrated that the gba1 deficiency mice showed gene regulation expression of the type I interferon. PMID: 27175482
    5. Rab7 accumulated in GCase deficient cells, supporting the notion that lysosomal recycling is impaired. Since recombinant GCase can reverse ALR impairment, we anticipate that strategies to restore GCase activity in the brains of both sporadic patients with PD and those with GBA1 mutations will improve autophagy lysosomal pathway, preventing the accumulation of a-synuclein and spread of pathology. PMID: 27378698
    6. heterozygosity for a Gaucher disease-associated mutation in glucocerebrosidase interferes with alpha-synuclein degradation and contributes to its accumulation PMID: 25351739
    7. Data indicate that ABC transporter A family member 12 knockout (Abca12(-/-)) epidermis had 5-fold more beta-glucocerebrosidase (GCase) protein, and a 5-fold increase in GCase activity. PMID: 24293640
    8. These results demonstrate, for the first time, a novel function of GBA1 as a beta-ChlGlc-synthesizing enzyme. PMID: 24211208
    9. Substrate compositional variation with tissue/region and Gba1 mutations in mouse models--implications for Gaucher disease. PMID: 23520473
    10. GBA1 and GBA2 activities had characteristic differences between the studied fibroblast, liver and brain samples. PMID: 22659419
    11. results not only point to a fundamental role for GBA in immune regulation but also suggest that GBA mutations in GD may cause widespread immune dysregulation through the accumulation of substrates PMID: 22665763
    12. This study suggested that several leads connecting GBA1 mutations with alpha-synuclein misprocessing have emerged as potential targets for the treatment of GBA1-related synucleinopathies. PMID: 22327140
    13. IFG stabilizes GCase in tissues and serum and can reduce visceral substrates in vivo. PMID: 22167193
    14. Mutations in GBA1 can cause Parkinson disease-like alpha-synuclein pathology; rescuing brain glucocerebrosidase activity might represent a therapeutic strategy for GBA1-associated synucleinopathies. PMID: 21730160
    15. evidence for the involvement of deletion of the GBA1 gene in multiple cell lineages in nonneuronopathic type 1 Gaucher disease PMID: 20962279
    16. The saposin C deficient mice backcrossed to point mutated GCase mimics the central nervous system phenotype and biochemistry of some type 3 (neuronopathic) variants of Gaucher disease. PMID: 20047948
    17. isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase PMID: 20148966
    18. Saposin C has multiple roles in glycosphingolipid catabolism and functions in Central Nervous System independent of its role as an stabilizer of GCase. PMID: 20015957
    19. mRNA shows generalized low level expression early in gestation with gradual appearance of differential expression appearing around gestational age E14 and significantly increasing at term and into adulthood. PMID: 11749048
    20. Results indicate that glucocerebrosidase deficiency, even in the absence of large amounts of sphingolipid storage, can trigger an inflammatory reaction. PMID: 11994410
    21. data indicate that saposin C is required for acid beta-glucosidase resistance to proteolytic degradation in the cell PMID: 12813057

    顯示更多

    收起更多

  • 亞細胞定位:
    Lysosome membrane; Peripheral membrane protein; Lumenal side.
  • 蛋白家族:
    Glycosyl hydrolase 30 family
  • 數據庫鏈接:


主站蜘蛛池模板: 又粗又大又黄又爽的免费视频| 性欧美熟妇videofreesex| 久久久久久久久无码精品亚洲日韩| 久久婷婷五月国产色综合 | 一本一道波多野结衣av一区| 性欧美激情aa片在线播放| 成人精品一区日本无码网站 | 国产成人久久久精品二区三区| 国产麻传媒精品国产av| 日韩激情无码不卡码| 亚洲熟女乱色综合亚洲图片 | 免费高清av一区二区三区| 国产好吊看视频在线观看| 亚洲精品伊人久久久大香| 怡红院av一区二区三区| 男女作爱免费网站| www国产亚洲精品久久麻豆| 日韩精品无码中文字幕一区二区 | 成人网站www污污污网站| 97高清国语自产拍| 图片小说视频一区二区| 18禁成年免费无码国产 | 国产成+人+综合+亚洲 欧美| 亚洲国产精品无码久久久不卡| 97久久精品人妻人人搡人人玩| 国产av无码专区亚洲awww| 亚洲精品无码久久千人斩| 一色屋精品视频在线观看免费| 玩弄人妻少妇老师美妇厨房| 免费吃奶摸下激烈视频| 99久久国语露脸精品国产| 黄网站免费永久在线观看下载| 国产在线视频主播区| 国产精品久久久久久超碰 | 黑人巨大精品欧美| 久青草无码视频在线观看| 国产成人亚洲综合青青| 99国产揄拍国产精品人妻| 国产特级毛片aaaaaa高清| 俄罗斯美女真人性做爰| 麻豆果冻精东九一传媒mv|